sudden cardiac death and malignant arrhythmias the scope ... perry 11 15 am.pdfjanuary 14-15, 2011...

January 14-15, 2011 SCA Conference 1

Sudden Cardiac Death and Malignant ArrhythmiasSudden Cardiac Death and Malignant ArrhythmiasThe Scope of the Problem in ACHDThe Scope of the Problem in ACHD

James C. Perry MDUC San Diego/Rady Children’s Hospital

EP and Adult CHD Programs

Arrhythmia, SCD in ACHDhere’s a starting point…

Oechslin et al. Am J Cardiol 20002609 ACHD pts: 199 deaths (~8%) - sudden death (26%) and CHF (21%)CoA (EF<40%) Ebstein (not WPW) ccTGA (EF<45%) AoV abnl TOFCoA (EF<40%), Ebstein (not WPW), ccTGA (EF<45%), AoV abnl, TOF

We haven’t understood this population very well…

Since late 1990’sSince late 1990 s, more CHD patients over age 18 than under in the U.S.

Now ~ 1,000,000 ACHD’ersNow ~ 1,000,000 ACHD’ers


The SCD Problem in ACHD is a ContinuumThe SCD Problem in ACHD is a Continuum

anatomyanatomygeneticsgenetics

Early events dictate and predict, to some extent,

AGEAGETIMETIME

surgicalsurgicali t tii t ti

hemodynamicshemodynamics

p , ,future risk of arrhythmia

and SCD

interventioninterventionyy

All of this leads to a substrate for late arrhythmia.“30 years old” may be young, but it also means 25+ years

exposure to post-op hemodynamics


“Dyssynchrony”BBB, AVB, IVCD

Categorizing the many different anatomies of ACHD:Heart Failure leads to VT/SCD

Systemic LV FailureSystemic LV FailureSystemic RV FailureSystemic RV Failure Subpulmonary RV FailureSubpulmonary RV FailureAI, CA abn’l,

failed RV-LV interaction (TOF, Ebstein’s)

ccTGA, HLHS,Mustard/Senning,

other Fontan

TOF, DORV

tachyarrhythmiay y

Sudden Cardiac Death

“Old-style Fontan” RA-PA connection

Current surgical management of “single ventricle physiology”

1- Ao-Pulm shunt: imposes a volume load on the ventricle (“chronic” stretch)

2 - Bidirectional Glenn shunt:reduces volume, persistent l f dilow sats for myocardium

3 - Fontan:multiple suture lines in atria, atrial stretch


Atrial reentry: Fontanavg 18avg 18YEARSYEARS

Fontan Fontan physiologyphysiology

18

Atriotomy, Atriotomy, bafflebaffle

RA stretch, RA stretch, obstruction, TRobstruction, TR

• Weber (AJC, 1989) MIX at 10 yrs = 50%50%• Gelatt (JACC, 1994) APC at 4½ yrs = 29%29%

TCPC = 14%14%RV-PA = 18%18%

C h (AJC 1995) APC 7 60%60%

Many/most Fontan patients develop atrial

tachyarrhythmias.• Cecchin (AJC, 1995) APC at 7 yrs = 60%60%

LAT +/- FEN = 30%30%• Nürnberg (ATS, 2004) early ECF/LAT - 11%, 38%11%, 38%• Bartz (JACC, 2006) heterotaxy, 4 yrs = 50%50%• Giannico (JACC, 2006) ECF at 15 yrs = 15%15%• Blaufox/PHN (JTCVS, 2008) mixed = 9.6%9.6%• Stephenson/PHN (JACC, 2010) all = 7.3%7.3%

Possibly decreasing incidence?

Systemic SV FailurePiran et al. Circ 2002;105:1189long-term f/u of 188 adult CHD

Adult Fontan patients:

45% had sx’s of tachyarrhythmia or obvious CHF18% died in 16 year follow-up18% died in 16 year follow-up33% died 2° to HF~90% of those dying with CHF had significant

arrhythmia, mostly IART, VT


Tetralogy of Fallot Subpulmonary RV Failure

“tetralogy”1. RV outflow obstruction

“monology”

A t i d i ti2. VSD3. Aortic override4. RVH

Anterior deviationof the infundibularseptum in utero

Reentry ventricular tachycardiaavg 27avg 27YEARSYEARS

TOF,TOF,DORVDORV

27

SCD/VT in TOF SCD/VT in TOF -- avg 27avg 27InfundibularInfundibular

incisionincisionPulm insuff,Pulm insuff,

RV enlargementRV enlargement

Subpulmonary RV failureP l it ti

5

10

15

20

25

30

35

ag

e y

rs

gg

Pulmonary regurgitation contributes to RV failure (Frigola 2004)correlates with RV size, QRSd and VTs (Gatzoulis Lancet 2000, Abd El Rahman 2002)

QRS durationmay be assoc. with VT (Gatzoulis 1997)improve after PVR (Therrien Circ 2002) - RVEDV 180ml/m2

Infundibular incisioncreation of isthmus-TA, VSD, PV (ablation data, Brigham, 2006)

0

1975 1980 1985 1990 1995 2000 2005 2010


VT in TOF

Counterclockwise VT circuit around the anastomosis of theanastomosis of the RV and conduit.

Successful RF line created, posterior aspect of

t i danastomosis and the tricuspid annulus.

Extreme bradycardia, sinus arrest on cessation of rapid atrial tachycardia. 25 yo with complex pulm atresia,

failed 1 1/2 ventricle approach, atriopulmonary Fontan


Bradycardia-induced VFconverted by AED at a gym


Systemic RV Failure - Atrial Switch (Mustard, Senning)d-transposition of the great arteries

One operation. Good early result.

Long-term:Long atrial suture lines, conduction blockSystemic right ventricle

Bradycardia/Tachycardiasd-TGA: Mustard, Senning

avg 25+avg 25+YEARSYEARS

dTGAdTGA

AtriotomyAtriotomy obstructionobstruction

25 + ⇒

NSR AtrTachyAtriotomy, Atriotomy, bafflebaffle

obstruction, obstruction, TRTR

BradycardiaBradycardia• “loss of sinus rhythm” - 20-60% at 20 yrs• pacing and HF

NSR AtrTachy

Atrial reentryAtrial reentry• most common tachy; can also 1:1 w/ ischemia, VT• rare paroxysm of adv AV block• uncontrolled IART: higher mortality riskuncontrolled IART: higher mortality risk

• pacing and HF• effects of slow HR on vent funct not well-studied• dilation due to dilation due to increased stroke volume increased stroke volume contributes to HF, VA’scontributes to HF, VA’s

11°° VT/SCD VT/SCD • Most ACHD w/ dTGA have fixed or reversible CA perfusion defects (systemic RV/coronary supply)• worse VT/SCD risk if Mustard with VSD• 70% have HF symptoms, poorly Rx’d• VT/VF documented in ~ VT/VF documented in ~ allall SCDSCD, ~ 80% with exercise


Systemic RV Failure:cc-TGA & ventricular tachycardiaavg 45avg 45

YEARSYEARS

Systemic RV Systemic RV cccc--TGATGA

30-40

Surgical Surgical interventionintervention

coronary insuff,coronary insuff,RV enlargementRV enlargement

Coronary insuff: Hornung. Heart 1998.Pts with isolated cc-TGAAll w/ fixed perfusion defects, most w/ reversible defects

CHF and SCD: (age 45) Graham; PiranCHF in 67% with, 25% without assoc lesions>25% early sudden death, >1/2 with CHFVT/VF common w/ CHF - what’s a normal RVEF?

Only a 3 yr old had a normal EF

Late AV Block

20-40

~ 30 YEARS~ 30 YEARS

Anatomy, Anatomy, geneticsgenetics

“Natural” late AVB“Natural” late AVB: ccTGA: ccTGAsurgicalsurgical

interventioninterventionhemodynamicshemodynamics

Previous Previous “transient” surgical AVB“transient” surgical AVB• 288 TOFTOF pts, operated 1950-60’s• 9% late sudden cardiac death• Avg return of conduction in late SCD

• Risk of acquired AV block: 2%/yr, 7%/yr with VSD

Huhta et al. Circ 1983DILV, lDILV, l--TGA (postTGA (post--op Fontan)op Fontan)• Risk of increasing degrees of AV

conduction abnormalities over timeg

was POD 7.3• Poor hemodynamics, later age at repair• 7/7 discharged in SCAVB diedSome with return of conduction 3Some with return of conduction 3--9 days 9 days

can have late SCD (AVB)can have late SCD (AVB)Hokansen, Moller. Am J Cardiol 2001


New AV block

• 57 yo Fontan, complex anatomy, AV-VA discordance

• 1st ECG in September

• 2nd ECG in October

Bradycardia and AVB-induced VT/VF


Ventricular tachycardia:LV failureavg 40avg 40

YEARSYEARS

Systemic Systemic LVLV

40

Surgical Surgical interventionintervention

aortic insuff,aortic insuff,LV enlargementLV enlargement

AI and decr LV function after Ross operation: >15% require re-op for AIup to 30% may have early and/or late VTdirect relationship between LVEDD and VA’s

RV-LV interaction: ***~10-15% of late post-op TOF patients will have reduced LVEF.Related to: 1) prior long duration shunt

2) aortic insufficiency3) RV enlargement, reduced RVEF, RBBB

Incidence of VA’s is higher in those with reduced LVEF


Dyssynchrony

Adult HF: (CRT) Bradley et al. JAMA 2003;289;7301634 pts, meta-analysis: CONTAK, MUSTIC, MIRACLE, InSync ICD

LBBB 54 87% LVEFs ≤ 30%; Ischemic CM 37 69%;

22°° to Bundle Branch Blockto Bundle Branch Block

LBBB 54-87%, LVEFs ≤ 30%; Ischemic CM 37-69%; Age ~ 65 yrs

ACHD: RBBB - operations: TOF, VSD, etc, etcLBBB - operations: AS, subAS, some DORV, AV canal

Adult pts: DAVID Trial (2002); MOde Selection Trial (2003)22°° to Pacingto Pacing

⇒⇒ Over 1 year+Over 1 year+Mortality, hosp and atrial fib > with more RV pacing

Ped/ACHD pts (mult studies)incr. RV paced QRS duration and age = poorer LV functionLocation: septum better than apex - LV dP/dt and LVEDP“Upgrade” from to DDDR: Ventricular funct worse

⇒⇒ Over 1 year+Over 1 year+

⇒⇒ Over decades?Over decades?

The SCD Problem in ACHD is PersonalThe SCD Problem in ACHD is Personal


Cumulative Personal Risk:Representative life of an ACHD pt (TOF, early SCAVB)

C C/EP C/EPC

cryo TVplasty cryo

Surg AVBEpi PM

gen ∆

leadsgen ∆

gen ∆ gen ∆

leadsgen ∆

leadsgen ∆

gen ∆

leadsgen ∆

1/126/12 10 25 40 55-60

shunt

“repair”

RVOTy

RVOT cryoRVOT

yRVOT

Age

19

The SCD Problem in ACHD is ChangingThe SCD Problem in ACHD is Changing


Changing… increasing complexity of the ACHD population

ASD/VSD

TOF

Mustard/Senning

Fontan2010

HLHS

Truncus

ASD/VSD

TOF

20 30 40 50 60

Mustard/Senning

Fontan

HLHS

Truncus

20 30 40 50 60

2020

Th P bl fTh P bl fThe Problem of The Problem of Lifetime Arrhythmia Burden Lifetime Arrhythmia Burden

in ACHD needs to be Quantifiedin ACHD needs to be Quantified


CRM needs by CHD type

DEFECTIncidence (in CHD

Population)Patent Ductus Arteriosus 7 3%

Estimate candidates:

DEFECT CRM needs pediatric

CRM needs ACHD

Patent Ductus Arteriosus 0.0% 0.0%Patent Ductus Arteriosus 7.3%Pulmonary Stenosis 6.6%Aortic Coarctation 6.2%

Ventricular Septal Defect 30.7%Atrial Septal Defect 8.9%

Total Anomalous Pulmonary Venous Return 1.8%

Atrioventricular Canal 4.4%Aortic Stenosis 4.0%

Truncus Arteriosus 1.9%Pulmonary Atresia 2.4%Tetralogy of Fallot 4.9%

Double Outlet Right Ventricle 1.7%

Patent Ductus Arteriosus 0.0% 0.0%Pulmonary Stenosis 0.0% 0.0%Aortic Coarctation 0.0% 6.0%

Ventricular Septal Defect 2.5% 2.5%Atrial Septal Defect 6.0% 25.0%

Total Anomalous Pulmonary Venous Return 2.0% 5.0%

Atrioventricular Canal 5.0% 8.0%Aortic Stenosis 5.5% 38.0%

Truncus Arteriosus 34.0% 26.0%Pulmonary Atresia 8.0% 29.0%Tetralogy of Fallot 28.0% 42.0%

Double Outlet Right Ventricle 14.0% 42.0%H l ti L ft H t 14 5% 49 0%

Pacing: AV block, SND/AT

ICD: primary,

secondaryHypoplastic Left Heart 4.0%Transposition of the Great

Arteries 6.3%

OTHER - Single vent, ccTGA, heterotaxy, Ebstein's 9.0%

Hypoplastic Left Heart 14.5% 49.0%Transposition of the Great

Arteries 34.0% 100.0%

OTHER - Single vent, ccTGA, heterotaxy, Ebstein's 32.0% 75.0%

secondary

CRT for HF

Pediatric CHD: overall 9%Adult CHD: overall 20%

< 10% are getting CRM Rx

The SCD Problem in ACHD needs a PlanThe SCD Problem in ACHD needs a Plan


PREVENTIONDyssynchronyBBB, AVB, IVCD

RESYNCH (CRT)EARLIER SURGERY?

Systemic LV FailureSystemic RV Failure Subpulmonary RV FailureAI, CA abn’l,

failed RV-LV interaction (TOF, Ebstein’s)

CCTGA, HLHS,Mustard/Senning

TOF, DORV

tachyarrhythmia

EARLIER INTERVENTION

RECOGNITION

NEW “NYHA”?

SURGERYCATHEPMOLECULAR Rx?

y y

SCD, morbidity

2° THERAPIES:ICDRESYNCHMEDS

Take home messagesHeart Failure and Arrhythmia in ACHD:

ACHD is an electroanatomic defect

• Important component of antiarrhythmic therapy inImportant component of antiarrhythmic therapy in ACHD patients with HF is heart failure therapy.

• Anticipate EP needs by each CHD defect, physiology

• Use all available opportunities, don’t burn bridgesUse all available opportunities, don t burn bridges

• Give the pediatric CHD patient a realistic appraisal of life as an adult CHD patient.

sudden cardiac death and malignant arrhythmias the scope ... perry 11 15 am.pdfjanuary 14-15, 2011...

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